Developmental problems of upper limb

Dr. Ip Wing-yukQueen Mary Hospital

The University of Hong Kong

Developmental disorderLesions pertaining to development• Congenital lesions that are not obvious at birth

- bony lesions- CNS lesions- vascular lesions

• Non-congenital lesions that are complicated by growth- post-traumatic conditions- inflammatory joint disease

Topics for today• Bony lesions: Madelung’s deformity,

multiple exostosis, pseudoarthrosis of upper limb, developmental radial head dislocation, radio-ulnar synostosis, fibrous dysplasia

• Soft tissue lesion: vascular malformation• CNS lesion: cerebral palsy• Inflammatory joint disease: Juvenile

inflammatory arthritis

Medelung’s deformity• Growth disturbance of the palmar and ulnar

portion of the distal radius physis, may involve whole radius

• Scaphoid facet and radial styloid normal• Excessive radial and palmar angulation

of distal radius• Abnormal palmar ligament tethering the

lunate to radius, proximal to physis(vicker’s ligament)

• Dominant inheritance of Leri-Weill dyschondrosteosis, mutation of SHOX gene

• Repeated loading with physeal injury may mimic the condition

Clinical features• Distal ulnar is dorsally

prominent• Distal radius appears to have

dorsal concavity in lateral view• Shorter forearm• Usually minimal functional

impairment so most do not need treatment

• Pain caused DRUJ instability may limit function

Indication for surgical intervention

• Cosmesis: physiolysis with release of abnormal ligament

• Before skeletal maturity

Indication for surgical intervention

Pain: Options:- dome osteotomy+ ligament resection- radial closing osteotomy+ ulnar shortening- radial opening osteotomy- radial osteotomy+ Sauve-Kapandji procedure or other DRUJ procedures

Hereditary multiple exostosis• Disorder of enchondroal plate,

osteochondromata grow from physis of long bone

• Autosomal dominant,mutated gene for formation of EXT protein, which is an enzyme for synthesis of heparin sulphate

• Longitudinal and angulatory growth abnormality with forearm deformity

Types • 1. main osteochondroma in distal ulna: short ulna

and bowed radius• 2. main osteochondroma in distal ulna, short ulna

and bowed radius, radial head dislocation2a. Osteochondroma on proximal radial metaphysis2b. No proximal radial osteochondroma• 3. main osteochondroma is in distal radius and

the radius is relatively short

Indication for surgical treatment• Lumps causing local pain, nerve

compression• Impairing forearm rotation• Visible deformity and discrepancy of

growth between radius and ulna• Radial head dislocation• Malignant transformation 1-5%

Types of procedures• Simple excision• Distal radial hemi-epiphyseal stapling• Sauve-Kapandji procedure• Radial head excision• Differential forearm lengthening-

osteotomy, fixation, ulnar lengthening, angular correction, radial head reduction

5 Steps to manage complicated forearm osteochondromata

• Resect osteochondroma• Correct ulnar bowing to increase

interosseous space• Correct distal radial tilt• Correct length discrepancy

between bones• Reduce radial head

subluxation/dislocation

Radio-ulnar synostosis• Congenital: fibrous, bony, associated with

radial head dislocation• Post-traumatic: in proximal forearm, mid-

forearm, distal forearm• Sequlae of Monteggia fracture, closed head

injury with heterotopic ossification• Loss of rotation and progressive radial head

subluxation

Indication for surgery• Not able to compensate by other mobile

joint in upper limb with functional problems• Various methods to separate synostosis

usually fail• Osteotomy for better position

Fibrous dysplasia• Abnormal bone growth where medullary

cavity is replaced by fibrous tissue• Abnormal bone growth or swelling of

bone• Most are monostotic, asymptomatic and

identified incidentally• Most can be treated with observation• Symptoms: pain, irregular bone growth,

bone deformity, one fracture• No known cure

Pseudoarthrosis of radius and ulna• Associated with neurofibromatosis• A segment, usually ulna is replaced by

fibrous tissue• Autosomal dominant• Progressive forearm deformity,

forearm short as one bone bears all the load, causing radius bowing,

radial head dislocation• Diminished rotation• Pain, instability, weakness

Surgical treatment• Achieve bone union, stabilize DRUJ, UCJ,

allow continued bone growth• Creation of 1 bone forearm• External distraction technique combined

with compression at pseudarthrosis site• Resection of pseudathrosis + vascularized

fibular grafting more reliable

CNS conditions• Cerebral palsy: a group of permanent

movement disorders that appears in childhood, caused by abnormal development or damage to the parts of the brain that control movement, balance and posture

• Post-cerebral vascular accident eg. AVM• Brain damage after birth

Organic Brain Damage

Pyramidal system Extra Pyramidal system

Loss of voluntarycontrol of some muscles

Loss of inhibitorycontrol of reflex arc Abnormal pattern

In coordination Slow speed

Spasticity Vicious cycle of muscle imbalance(Shortening & weakening of tonic muscles,Lengthening & weakening of phasic muscles

Static & dynamic deformity of bone & soft tissues, which is specific for the brain damage

Hand control is by cotico-spinal pathway

Classification based on motor function

• Spastic type• Non-spastic type- dyskinetic- ataxic- hypotonic- mixed

Typical deformity in spastic type• Shoulder : adduction, internal rotation• Elbow : flexion • Forearm : pronation• Wrist : palmar flexion, ulnar deviation• Fingers : flexion/swan neck deformity• Thumb : thumb in palm deformityThe proximal joints are for positioning of the functional part, i.e. the hand

The deformity seems typical, butbrain damage is very individual

Typical deformity

Spasticity• Acts like a brake on the system

& reduces speed of movement, increases energy consumption

• Inhibits voluntary control• Shortened muscle works in

ascending part of length/tension curve – lead to weakness

• Lengthened muscle works in descending part of length/tension curve – lead to weakness

- Muscle control/relaxation techniques- Splints to reposition part, modify muscle strength- Electrical stimulation of antagonistic muscles - Neurosurgical procedures: fasiculotomy of peripheral

nerves- Pharmacologic agents: baclofen - Botulinum-A toxin injections - MUSCULOSKELETAL OPERATIONS

Treatment of spasticity

Surgical indications- Voluntary use of hand

during activities, with good potential for functional improvement

- Deformity interferes with function- upper limb, gait hygiene, discomfort

- (cosmesis)

Pre-op Evaluation• Each patient has different degree of CNS

lesion and treatment should be individualized• Multiple examination: motor, sensory,

intelligence, social (family support, parental stress, home environment, peer group)

• Pattern of movement disorder: spastic, dyskinetic, ataxic or mixed

• Classification: Manual ability Classification system; House Functional Classification; Zancolli’s classification of active finger and wrist extension; Modified House classification of thumb-in-palm deformity

Pre-op Motor Evaluation of UL• Observation of activities• Whole upper limb examination rather than

individual muscle• Pattern of hand usage • Speed of movement• How much is the imbalance of

agonist & antagonist?• How good is the voluntary control,

any mirror movement• Potential muscle for transfer: spasticity, power,

voluntary control • EMG study: phasic activities, recruitment pattern• Fixed deformities

Evaluate grasp & release with wrist flexed, wrist neutral, wrist extended

GR Zancolli, JHS 1983

I Finger extension with wrist in neutral or less than 20º of flexion

IIA Active wrist extensors present. Finger extension only with wrist in more than 20º of flexion

IIB No active wrist extensors present. Finger extension only with wrist in more than 20º of flexion

III Finger extension not possible even with maximal wrist flexion

Zancolli classification of Hand & wrist

Botulinum A toxin (Botox) injection

Facilitates surgical decision for patient & surgeon

Before & after Botox injection to intrincics

Timing of surgery• Consider better cerebral plasticity at

younger age, less neglect• Need to understand & comply with

rehabilitation programme• Staged procedures are frequently required• Clear evaluation of functional use of hand is

possible (age 6-12)• Adult can still be benefited if there is all

along some functional use

Surgical goals

• Improve quality of life: function, cosmesis, comfort, hygiene, gait

• Improve upper limb function: range of movement, strength & endurance

• Poor helping hand to better helping hand in patient with 1 hand more severely involved

Musculo-skeletal surgery • Only deal with spasticity & its sequalae• No way to deal with dystonia & sensory

deficit• Restore muscle balance around joints• Achieve best sacromere length • Achieve stable joint in functional position• Control pain by decrease spasticity, better

hygiene by better posture, improve gait with better bilateral upper limb positioning

Surgical interventionShoulder External

rotated & abducted for ADLElbow Extended

Forearm Neutral rotation

Wrist Neutral flexion

Digits Extended out of palm

Surgical planning

• Address the spastic agonist

or augment the antagonist to achieve balance at a joint

• Proximal to distal, extrinsic to intrinsic proximal surgery may alter distal pattern of

spasticity: Correct of wrist flexion deformity may affect digital positioning (further digital flexor tightness)Swan neck deformity after long flexors lengthening

• Do not use Classification patterns to make surgical decisions

•Avoid reverse deformity

Surgical optionsProcedures Shoulder

AdductionElbow flexion

Forearm pronation

Wrist flexion/UD Thumb in palm Finger deformity

Release Bicipital aponeurosis

PT releasePQ release

Flexor/Pronator fascial release

1st web Z plasty adductor release 1st DI release

Intrinsic release at origin/insertion

Lengthening PM lengtheningSubscapularis lengthening

Biceps lengtheningBrachialis lengthening

PT lengthening FCU lengtheningFCR lengthening

FPL lengthening FDS, FDP lengthening

Tendon transfer

LD transfer to poster-lateral humerus

PT re-routingFCU → ECRB

BR→ECRL/BECU → ECRL/BFCU → ECRL/BFCR → ECRL/BPT → ECRL/B

PL→APLPL→EPLPL→EPBFCR→APLBR→EPBBR→EPLEPL re-routingACC APL →EPB

FCU → EPC BR → EDC1st band re-routing FDS tenodesis

Bone/joint Stabilization

Rotational osteotomy

Wrist fusion with PRCPRC

MCPJ fusionMCP capsulodesisThumb IPJ fusion

DIPJ fusionVolar plateCapsulodesisPIPJ fusion

Shoulder • Target : improve space of movement of upper

limb, improve positioning of hand, improve hygiene, better gait

• Key muscle imbalance: spastic Pectoralis major

• Operative procedure: Fractional lengthening of PM, Z-plasty of Subscapularis

Fractional lengthening of Pectoralis Majorto gain passive abduction & external rotation

Elbow• Target: improve positioning of forearm & hand

(extension), improve cosmesis by ↓ flexor tone

• Typical deformity : elbow flexion• Key muscle imbalance : spastic biceps &

brachialis Vs. normal/weak triceps• Operative procedures : Biceps fractional /z

lengthening, Brachialis lengthening, release of bicipital aponeurosis, fascial release of commom flexor origin

• Manske 2001: 49˚improvement in flexion posture angle, active extension 43˚to 27˚, use of arm ↑from 12% to 44%

Workhorse procedure: Bicipital aponeurotic release, flexor aponeurotic

release till elbow can be extended passively

Forearm• Target : some supination in at least 1 forearm, to

visualize thumb during ADL, prevent radial head dislocation which results in loss of rotation & pain

• Operative procedures : PT lengthening to decrease pronator tonePT release when supination short of neutralPQ release for patient with tight passive supinationPT re-routing for patient with no active supinationFCU → ECRB transfer has a supination component

• Bunata et. Al.2006: PT re-routing ↑65˚supination• Cheema et.al. 2006: FCU to ECRB more effective

to produce supination than PT re-routing or BR re-routing

Workhorse procedure: Forearm rotation• Fractional Pronator

teres lengthening at musculo-tendinous junction

• Partial relaease of PQ at origin

• FCU transfer to ECRB, which deals with wrist extension & has a supination action

Wrist• Target : active wrist extension to functional

position, improve cosmesis• Typical deformity : flexion/ulnar deviation

Static : fixed flexion contracture of > 45°Dynamic : active extension to 45 ° less than neutralFunctional : full active range, but as spastic flexors

fire, wrist pulled into flexionKey muscle imbalance : weak wrist extensors,

tight/or spastic wrist flexion ± finger flexors2 ° capsular contracture

Finger flexion deformityTarget: Hand opening with wrist neutral

improve cosmesisOperative procedures : fractional lengthening of

FDS/FDP up to full extension with wrist in neutralFCU, BR → EDC

Workhorse procedure for wristFor patient with active finger extensor FCU transfer to ECRBTension transfer at 0-5゜ flexion

Fractional lengthening of long flexors till digits can be extended passively

Fingers

For patients with no active finger extensor

• FCU/BR transfer to EDC

Pre-op Vs Post-op

Pre-op Post-op

Wrist fusion for motionless hand to improve hygiene & cosmesis, make a

stable wrist for stabilizing objects

Swan Neck deformityTarget : • Stable PIPJ• Faster & smooth hand

closing• Improve cosmesisKey muscle imbalance : intrinsic

spasticity• intrinsic tightness unmasked

after long flexor release

Many operative procedures

• Intrinsic release at origin or insertion• Lateral band re-routing• FDS tenodesis• Central slip tenotomy • Spinal oblique retinacular ligament

reconstruction • PIPJ fusion• PIPJ volar plate capsulodesis• DIPJ fusion

Partial intrinsic release at originimprove in resting posture

Lateral band to volar plate to achieve balance

Thumb in palm deformityTarget-Improve thumb positioning during fisting

-Improve thumb abduction during hand opening

Operative procedures : - ↑1st web space by Z plasty- Identify the muscle imbalance

decrease the tone of spastic muscle, augment the weakened muscle to achieve balance - Achieve good resting posture by tenodesiseffect, or bony realignmemt with arthrodesis

Intrinsic release: FPB release , Adductor pollicis release at origin

APL tenodesis to FCR & ECRL as sling of the thumb for good

resting posture

Post-op Rehabilitation- Cortical control of motor unit is specific- Tendon transfer changes the whole system- The motor system must re-learn motor program to fully utilize the release/transfer

• Splint program according to surgical details

• Protect transferred tendon

• Prevent adhesion or rupture.

Vascular Tumours

• Complex classification• Malignant ones extremely

rare• Benign ones might be

locally aggressive and difficult to treat

• Gradual increase in size after birth

Non-neoplastic vascular malformations

• Slow flowcapillary, venous, lymphatic

• High flowarterial, AV fistulae, AV malformations

• Complex combine

Problems in the upper limb

• Increase size of limb• Functional impairment due to progressive in

size, or progressive tissue ischaemia• Signs of congestive heart failure• Palliative Vs curative surgery

- Embolization with wide excision +/- flap coverage, or amputation may be required

Inflammatory Arthritis affecting the growth of musculo-skeletal

system• Juvenile Idiopathic Arthritis(JIA) –used most by

paediatric rheumatologist• Malfunctioning of immune system, unknown

cause• Disease pathology similar to adult counterpart• Involve 1 or many joints, with fever, rash,

inflammation of internal organs• Oligoarticular: < 5 joints or polyarticular

International League of Association for Rheumatology

Classification of JIA

• Systemic-onset• Persistent oligoarthritis• RF-ve polyarthritis• RF+ve polyarthritis• Psoriatic • Enthesitis-related• Undifferentiated

Treatment • Multi-disciplinary approach• Control symptoms, prevent joint damage,

maintain function• Mainstay is medical: NSAIDs, DMARDs• Orthropaedic treatment : - solitary joint arthritis when disease is under

control, synovectomy to slow down the joint damage process

- Reconstruction of deformity like adult RA patients

Special consideration for operation in growing skeleton

• Synovectomy or tenosynovectomy to reduce synovial damage should be done earlier before joint erosion

• Joint stabilization should be done after skeletal maturity

• Prosthesis arthroplasty should be considered at a later age

Earlier synovectomy to slow down cartilage and bony damage

by synovitis

Tendon transfer procedures for extensor similar to adult RA

Try to preserve DRUJ, rather than excision arthroplasty

Keep auto tissue with excision arthroplasty of MCPJ rather than

using prosthesis arthroplasty

Thank you very much for your attention

wyip@hku.hk